磷酸化γ氨基丁酸γ2受体抗体
规格：1尘驳/1尘濒
英文名： phospho-GABRG2 (Ser366)
别名： p-GABAA Rγ2 (Ser 365); CAE 2; CAE2; ECA 2; ECA2; GABA(A) receptor subunit gamma 2; GABA(A) receptor subunit gamma-2; GABRG 2; GABRG2 antibody Gamma aminobutyric acid (GABA) A receptor gamma 2; Gamma a
分子量： 50kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthesised phosphopeptide derived from human
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep,
细胞定位：细胞膜
磷酸化γ氨基丁酸γ2受体抗体产物介绍：background: GAD-65 and GAD-67, glutamate decarboxylases, function to catalyze the production of GABA (gamma-aminobutyric acid). In the central nervous system, GABA functions as the main inhibitory transmitter by increasing a Cl- (chloride) conductance that inhibits neuronal firing. GABA has been shown to activate both ionotropic (GABAA) and metabotropic (GABAB) receptors, as well as a third class of receptors called GABAC. The ?subunit of GABAA receptors are important for benzodiazepine binding and modulation of GABA-mediated Cl- current. GABAA R? is a 467 amino acid mulit-pass membrane protein localized to the postsynaptic cell membrane. Present as a pentamer with other GABAA receptor chains (Alpha, Beta, Gamma, Delta and Epsilon), the GABAA ligand-gated Cl- channels selectively complex with D5DR to enable mutual inhibitory functional interactions between the two receptor systems. Defects in the gene encoding GABAA R Gamma 2 have been 磷酸化γ氨基丁酸γ2受体抗体found to be the cause of childhood absence epilepsy type 2, familial febrile convulsions type 8, generalized epilepsy with febrile seizures plus type 3 and severe myoclonic epilepsy in infancy. Function: GABA, the major inhibitory neurotransmitter in the vertebrate brain, mediates neuronal inhibition by binding to the GABA/benzodiazepine receptor and opening an integral chloride channel. Subunit: Generally pentameric. There are five types of GABA(A) receptor chains: alpha, beta, gamma, delta, and rho. Interacts with GABARAP. Subcellular Location: Cell junction, synapse, postsynaptic cell membrane; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein. Post-translational modifications: Palmitoylated by ZDHHC3/GODZ; which may affect presynaptic clustering and/or cell surface stability. DISEASE: Defects in GABRG2 are the cause of childhood absence epilepsy type 2 (ECA2) [MIM:607681]. ECA2 is a subtype of idiopathic generalized epilepsy (IGE) characterized by an onset at age 6-7 years, frequent absence seizures (several per day) and bilateral, synchronous, symmetric 3-Hz spike waves on EEG. During adolescence, tonic-clonic and myoclonic seizures develop. Some individuals manifest ECA2 occurring in combination with febrile convulsions. Defects in GABRG2 are the cause of familial febrile convulsions type 8 (FEB8) [MIM:611277]. A febrile convulsion is defined as a seizure event in infancy or childhood, usually occurring between 6 months and 6 years of age, associated with fever but without any evidence of intracranial infection or defined pathologic or traumatic cause. Febrile convulsions affect 5-12% of infants and children up to 6 years of age. There is epidemiological evidence that febrile seizures are associated with subsequent afebrile and unprovoked seizures in 2% to 7% of patients. Defects in GABRG2 are the cause of generalized epilepsy with febrile seizures plus type 3 (GEFS+3) [MIM:604233]. Generalized epilepsy with febrile seizures-plus refers to a rare autosomal dominant, familial condition with incomplete penetrance and large intrafamilial variability. Patients display febrile seizures persisting sometimes beyond the age of 6 years and/or a variety of afebrile seizure types. GEFS+ is a disease combining febrile seizures, generalized seizures often precipitated by fever at age 6 years or more, and partial seizures, 磷酸化γ氨基丁酸γ2受体抗体with a variable degree of severity. Defects in GABRG2 are a cause of severe myoclonic epilepsy in infancy (SMEI) [MIM:607208]; also called Dravet syndrome. SMEI is a rare disorder characterized by generalized tonic, clonic, and tonic-clonic seizures that are initially induced by fever and begin during the first year of life. Later, patients also manifest other seizure types, including absence, myoclonic, and simple and complex partial seizures. Psychomotor development delay is observed around the second year of life. SMEI is considered to be the most severe phenotype within the spectrum of generalized epilepsies with febrile seizures-plus. Similarity: Belongs to the ligand-gated ion channel (TC 1.A.9) family. Gamma-aminobutyric acid receptor (TC 1.A.9.5) subfamily. GABRG2 sub-subfamily. Gene ID: 2566 Database links: Entrez Gene: 282240 Cow Entrez Gene: 2566 Human Entrez Gene: 14406 Mouse Entrez Gene: 29709 Rat Omim: 137164 Human SwissProt: P22300 Cow SwissProt: P18507 Human SwissProt: P22723 Mouse SwissProt: P18508 Rat Unigene: 7195 Human Unigene: 5309 Mouse Unigene: 159942 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
磷酸化γ氨基丁酸γ2受体抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：神经生物学 通道蛋白 细胞膜受体 G蛋白偶联受体 G蛋白信号
储存条件： Store at -20 癈 for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid